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The retinoid X receptor (RXR)-agonist bexarotene and the histone deacetylase inhibitor (HDACI) vorinostat are each established monotherapies for cutaneous T-cell lymphomas (CTCLs). We investigated the combination of HDACI and retinoic acid receptor (RAR)/RXR agonists in vitro and in a phase I, multicenter, open-label, two-part dose-escalation study. The combination of bexarotene with a HDACI in vitro leads to cooperative activation of gene transcription and reduction of cell viability in human tumor cell lines. The primary clinical objective was to determine the maximum tolerated dose (MTD) of bexarotene plus vorinostat in 23 patients with CTCLs. The MTD for part I was established at vorinostat 200 mg/day plus bexarotene 300 mg/m2/day. The MTD for part II was not reached. Four patients had an objective response and seven patients experienced pruritus relief. We conclude that concomitant administration of vorinostat and bexarotene is feasible only if lower doses of each drug are administered relative to the product label monotherapy doses. [ABSTRACT FROM AUTHOR]

Tumor necrosis factor (TNF)-α antagonists have considerably improved the therapeutic approach to chronic inflammatory disorders including psoriasis vulgaris. Recently, some cases of highly aggressive hepatosplenic T-cell lymphoma (HSTCL) have developed in patients with inflammatory bowel diseases (IBD) being treated with infliximab or adalimumab. Analysis of the published data suggests that the emergence of HSTCL is favored by the combination of purine analogues and infliximab or adalimumab in the therapy of a granulomatous inflammation involving Vδ1+γδ T cells. Because psoriasis vulgaris is different from IBD in regard to the type of inflammation, the concomitant therapies used and the tissue-specific subsets of γδ T cells, the use of infliximab or adali-mumab in psoriasis may not necessarily be associated with an increase in the risk of HSTCL. [ABSTRACT FROM AUTHOR]

Radioimmunotherapy with Yttrium-90 (90Y) ibritumomab tiuxetan (IT) has been shown to be effective in systemic B-cell lymphomas. We conducted a pilot study to evaluate the outcome and assess complications of 90Y IT therapy in patients with primary cutaneous B-cell lymphomas (PCBCL). Ten patients, all but one, with relapsed PCBCL were included and treated with rituximab (250 mg m-2/body surface) on days 1 and 8 followed by a single dose of 90Y IT (11-15 MBq kg-1). The overall response rate was 100%. The complete response rate was 100%. The median time to relapse was 12 months. Ongoing remissions were achieved in four patients (median follow-up 19 months). Transient and reversible myelosuppression (grade 3-4) was the most frequent adverse event. Radioimmunotherapy with 90Y IT is an effective treatment in relapsed primary cutaneous follicle centre lymphomas and diffuse large B-cell lymphoma leg-type. Further investigations in controlled randomised clinical trials evaluating the role of 90Y IT versus rituximab in PCBCL are needed. [ABSTRACT FROM AUTHOR]

Background: Primary cutaneous lymphomas form a heterogenous group of lymphatic neoplasias.They manifest themselves on the skin and are the second most frequent group of non-Hodgkin lymphoma (NHL) following gastrointestinal lymphomas.The number of epidemiologic studies is small due to limited availability and limited comparability on population-based data. Patients and methods: In the present study the first evaluation of the German Central Registry for Cutaneous Lymphomas (ZRKL) of the German Society of Dermatology (DDG) is undertaken on the basis of 998 patients.The epidemiology of cutaneous lymphomas in Germany is compared to other national or regional lymphoma registries. Results: Based on the registration of 998 patients from 26 clinics in Germany,a clear predominance of cutaneous T-cell lymphomas (85 %) in comparison to cutaneous B-cell lymphomas (14 %) is seen.The most frequent representative of CTCL is mycosis fungoides,composing 62 % of cases with a slight predominance of men (M:F = 1.6:1).Differences are also seen in stage of the disease at first presentation of patients with cutaneous lymphomas.While, for example, 80 % of patients with mycosis fungoides in Germany present in early stages (I–IIA),in the USA 34 % of patients are in the tumor stage or have organ involvement at presentation. Conclusions: The ZRKL of the DDG for the first time presents epidemiologic data from Germany, allowing comparison with other nations for the study of etio-logical factors and socioeconomic influences. Further, the ZRKL supports the development of uniform and quality-oriented diagnostic criteria and therapeutic options. Finally, the ZRKL provides a foundation for future intensive study of clinical and scientific questions regarding cutaneous T- and B-cell lym-phomas. [ABSTRACT FROM AUTHOR]

Summary. An unusual course was observed in a patient with indolent T-prolymphocytic leukaemia (T-PLL) who subsequently developed mycosis fungoides (Mf), lymphomatoid papulosis (LyP) and cutaneous CD30+ anaplastic large cell lymphoma (ALCL). Polymerase chain reaction analysis demonstrated identical monoclonal T-cell receptor-β and -γ gene rearrangements in all the different clinical entities. Furthermore, cytogenetic studies revealed the same aberrant clone with trisomy of chromosome 8 in T-PLL and ALCL cells. This unique observation suggests that in T-PLL, the leukaemic cells might undergo secondary transformation, subsequently resulting in different phenotypes of cutaneous T-cell lymphoma. [ABSTRACT FROM AUTHOR]